Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019214 (hepatosplenomegaly)
 4,408 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A neonate presented with poor feeding, jaundice and hepatosplenomegaly. He was in liver failure with disseminated coagulopathy. Post-mortem liver biopsy revealed discrete granulomas with central necrosis and acid-fast bacilli on Ziehl-Nielsen's stain. The mother had active tuberculosis at delivery. The principal characteristics of congenital tuberculosis are reemphasized in this observation.
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PMID:Congenital tuberculosis. 849 20

There is limited information on HIV infection in children in West Africa. This prospective case series study was done to determine the size of the problem and the feasibility of selective screening for infection based on clinical presentation. It involved infants and other children admitted to the Children's Emergency Ward and Paediatric Medical Ward of the University of Maiduguri Teaching Hospital, Nigeria, from the beginning of September 1992 to the end of September 1994. Clinical evaluation followed by serologic tests (ELISA and Western blot techniques) was undertaken. Descriptive study; frequencies were compared using chi 2 test for Fisher's exact test as appropriate. One hundred and ninety nine (10.9%) of 1,822 admissions were screened. One hundred and fifty eight (79.4%) were ELISA negative and 17 (8.6%) ELISA and WB positive; a further 10 (5%) were ELISA positive but WB indeterminate and 14 (7%) were ELISA positive but WB negative in 12 or untested in two. All the infections were HIV-1. Sixteen (39%) patients (nine WB positive, three WB indeterminate and four ELISA positive only) are dead, 14 from HIV-related illnesses, two (4.9]) are alive and 23 (56.1%) lost to follow up; 11 of the HIV-related deaths involved infants. Presence of persistent diarrhoea, prolonged fever, oral thrush, hepatosplenomegaly, diagnosis of tuberculosis and severe malnutrition with gastroentereritis, and multiple (> 3) diagnosis on admission were significantly (p < 0.05) associated with WB confirmed HIV-1 seropositivity and could serve as indicators for selective screening. HIV-1 infection in hospitalised infants and children has become an important problem in Nigeria, presentation in infancy is associated with a high case fatality rate, and the practice of selective screening based on clinical presentation would appear to be feasible.
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PMID:Presentation and outcome of HIV-1 infection in hospitalised infants and other children in north-eastern Nigeria. 914 72

Seven children, six boys and a girl, aged from 2 to 15 years with proven myelofibrosis are reported. The clinical presentation in each of them was more or less similar with weight loss, moderate or low-grade fever, and abdominal distension with pain or discomfort for some months. They had hepatosplenomegaly. The spleens, enlarged to more than 6 cm below the costal margin, were smooth, firm and not tender. There was a variable degree of generalized lymphadenopathy. They were diagnosed as myelofibrosis associated with tuberculosis. The clinical response to anti-tuberculous chemotherapy was remarkable. Extensive search should be made for evidence of tuberculosis in children presenting with myelofibrosis.
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PMID:Tuberculosis and myelofibrosis in children: a report. 917 80

Tuberculosis continues to be a serious problem in public health even thought eh causative bacillus was discovered over 100 years ago. The authors present a clinical case that illustrates the current concern regarding the disease. The case concerns a child of emigrant parents, who are drug addicts and whose illness has not been fully ascertained. The child was hospitalised at three and an half months with fever, malnutrition, opisthotonos and hepatosplenomegaly. After the diagnosis of disseminated tuberculosis affecting the central nervous system, treatment was started with five antituberculosis drugs and with corticosteroids. Respiration improved favourably, but after 19 days the patient suffered a partial tonic-clonic seizure. Subsequently, hydrocephalus was observed and a shunt was applied. Bacteriological examination of the gastric aspirate showed a strain of Mycobacterium tuberculosis resistant to isoniazid and streptomycin. In the eight month of therapeutics, three antituberculosis drugs were still being administered, the shunt was still present and the patient showed a severe psychomotor retardation. The child belonged to a risk group and presented a serious form of tuberculosis with multidrug-resistance, illustrating that this group of children is particularly vulnerable and reflect transmission of this illness among adults.
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PMID:[Tuberculosis: a forgotten challenge]. 934 Oct 47

A unique feature of Mycobacterium tuberculosis is its ability to establish latent infection in the human host, which can reactivate to cause disease years later. In the present study, the mechanisms involved in the control of latent tuberculous infection were examined using two murine experimental tuberculosis models. Analysis of the model involving infection of mice with a relatively low inoculum of the virulent Erdman strain of M. tuberculosis indicated that in vivo inhibition of reactive nitrogen intermediate (RNI) production by the nitric oxide synthase inhibitor aminoguanidine resulted in reactivation. This reactivation was evidenced by hepatosplenomegaly, a robust tissue granulomatous reaction, and increased bacillary load. IFN-gamma, TNF-alpha, and inducible nitric oxide synthase were all expressed throughout the latent phase of infection. Reactivation of latent tuberculous infection by aminoguanidine treatment was confirmed using a second murine tuberculosis model based on treatment with antimycobacterial drugs. Results obtained using this drug-based model also suggested the existence of an RNI-independent antimycobacterial mechanism(s) operative in the latent phase of infection. Together, these data suggest that both RNI-dependent and -independent mechanisms contribute to the prevention of tuberculous reactivation.
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PMID:Effects of aminoguanidine on latent murine tuberculosis. 946 39

A 29-year-old Caucasian woman presented to hospital with a 2-day history of diarrhoea, anorexia and rigors. Investigations showed abnormal liver function tests, hyponatremia, hypoalbuminaemia and lymphopenia. The initial chest radiograph was normal. A bone marrow trephine biopsy showed non-caseating granulomata and she subsequently developed miliary shadowing on the chest radiograph. A transjugular liver biopsy confirmed the presence of acid-alcohol fast bacilli. Despite starting triple therapy for miliary tuberculosis she remained febrile and developed massive hepatosplenomegaly, jaundice and pancytopenia. Standard triple therapy was substituted with ethambutol, streptomycin and oral prednisolone and the patient made a dramatic recovery. The clinical symptoms of miliary tuberculosis are frequently non-specific and the onset of the illness is often insidious. The liver is involved in almost all patients with miliary tuberculosis, but massive hepatosplenomegaly and jaundice are rare. Standard triple-therapy should be discontinued when there is significant liver dysfunction, and corticosteroids should be considered for patients with miliary tuberculosis who fail to respond to conventional therapy.
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PMID:Massive hepatosplenomegaly, jaundice and pancytopenia in miliary tuberculosis. 957 Jun 66

We report a patient aged 41 years with fever of unknown origin. Notable aspects of his travel history were a trip to the Philippines and a sailing trip around Sicily. The patient presented with fever up to 40 degrees C since 4 weeks, weakness, headache, hepatosplenomegaly and night sweat. No specific cause could be found. Based on clinical findings tuberculosis was suspected and empirical tuberculostatic treatment was started. However, during the following 6 weeks the patient's condition deteriorated. A bone marrow biopsy performed to exclude a haematological malignancy revealed Leishmania sp. in macrophages. This histological diagnosis was confirmed retrospectively by re-examination of a previously performed liver biopsy and by an increased anti-leishmania serum antibody titer of 1:1280. The patient was treated with sodium stibogluconate (pentostam, 850 mg) for 30 days and recovered slowly.
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PMID:A case of visceral leishmaniasis in Austria. 962 27

51 cases of granulomatous hepatitis were seen among 1234 liver biopsies over a 10 year period. Tuberculosis was the commonest cause seen in 55 percent of cases. Other causes included leprosy, sarcoidosis, histoplasmosis, brucellosis, amoebic liver abscess, lymphoma and malignant granuloma. 12 percent of cases remained undiagnosed. Clinically these patients presented with pyrexia and hepatosplenomegaly. Jaundice was uncommon. Many showed elevated alkaline phosphatase levels, anaemia and raised ESR Granulomatous hepatitis of unknown aetiology with FUO was seen in 6 percent cases only.
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PMID:Granulomatous hepatitis: a retrospective study. 972 54

Miliary tuberculosis infection in childhood remains a significant health problem in developing countries. To characterize the clinical features of the disease, symptoms, signs and laboratory findings, associated infections and outcomes of the 23 children (14M, 9F) with miliary tuberculosis admitted to the Dicle University Hospital, Diyarbakir, Turkey from 1990 to 1997 were analysed. Mean age of the patients was 3.7 y ranging between 6 months and 11 y. All but 3 patients were below 6 y of age, and 5 of the patients were under 1 y of age. All of the children had not received BCG vaccination except for 1 who was immunized after the age of 5 y. Only 6 children had a positive reaction to 5 TU tuberculin test at admission to hospital. Eight of the 23 subjects were infected in the family, and all were nonHIV infected cases. All of the children were diagnosed clinically and by a miliary infiltrate visible on chest roentgenogram. Five cases had positive culture results for Mycobacterium tuberculosis. Duration of onset of symptoms prior to admission ranged between 15 d and 8 months with a mean of 58 d. The most frequently seen symptoms and clinical findings were fever in 17 cases, rales in 14, loss of appetite and weight in 10 and hepatosplenomegaly in 9 cases. Two cases had prior measles infection and 1 of the patients presented facial paralysis. Seven cases had also tuberculous meningitis. The outcome in general was good, with mortality in 2 cases.
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PMID:Miliary tuberculosis in children: a clinical review. 981 15

A women, 50 years old, has been observed for 4 years because of recurrent infiltrations in both lungs. Biopsy of those lesions revealed sarcoid-like granulomas. Tubercle baccilli were not present in those lesions nor in the sputum or bronchial washings. Anti-tuberculosis therapy was without effect. On admission to our hospital she was in good performance state. There was an infiltration in the base of the left lung. Hepatosplenomegaly was observed on USG examination. Aspartate aminotransferase was 49 UI/l, alanine aminotransferase 70 UI/l. Alkaline phosphatase was 167 UI/l and the titer of antimitochondrial antibodies was 1:2000. Primary biliary cirrhosis was suspected, but the patient refused liver biopsy. Prednisone in the dose of 60 mg per day was given to suppress the granuloma formation in the lungs. During this treatment there was a decrease in size of liver and spleen, lung lesion disappeared and the titer of antimitochondrial antibodies decreased to 1:40. After 3 months of treatment the dose of prednisone was reduced gradually. When she was receiving 15 mg of prednisone every other day the titer of antimitochondrial antibodies rose to 1:8000 and the activity of alkaline phosphatase to 448 UI/l. At this time she accepted liver biopsy. Primary biliary cirrhosis was diagnosed. The possible connection between sarcoid-like granulomas in the lungs and the primary biliary cirrhosis is discussed.
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PMID:[Primary biliary cirrhosis with sarcoid-like infiltrations in the lung]. 985 54


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